dss 2012 case 5 mohamed el hag duncan stearns michael coffey mark cohen
TRANSCRIPT
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DSS 2012 Case 5
Mohamed El HagDuncan StearnsMichael Coffey
Mark Cohen
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Clinical History
• 3 month old girl with a bump on the side of her head
• Previously healthy, normal prenatal ultrasounds
• Head circumference 44cm (>95th percentile)
• No neurological deficits
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MRI: T2 & T1 with contrast
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Dural-based,well-demarcated tumor
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Biphasic morphology
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Question for discussion:
Prognosis?(diagnosis already provided
by radiologist)
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Disclosure
Unfortunately, the authors have nothing to disclose, including the
answer to their question.
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GFAP SYN NF
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Desmoplastic infantile astrocytoma and ganglioglioma (DIA/G)
• Taratuto AL, et al. Superficial cerebral astrocytoma attached to dura. Report of six cases in infants. Cancer. 1984
• VandenBerg SR, et al. Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential ("desmoplastic infantile gangliogliomas"). Report on 11 cases of a distinctive embryonal tumor with favorable prognosis. J Neurosurg. 1987
• Presentation in infancy
• Macrocrania• Voluminous size• Cystic component• Divergent
differentiation• Desmoplastic
stroma• Good prognosis
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D(S)IA/Gs with anaplastic features
Case Age Features Outcome Follow-up
VandenBerg (87)Case #6
3m MFs, necrosis A, NR 2.7 yrs
Duffner (94)Case #3
4m MFs A, NR 5 yrs
Al-Sarraj (96) 8mMFs, Palisading
necrosisA, NR 3 yrs
Kuchelmeister (96) 9mMFs, MVP, necrosis
A, NR >2 yrs
DeMunnynck (02)* 2yMFs, MVP, necrosis
DOD 1 yr
Hoving (08) 7m MFs, necrosis DWD 9 mos
*multifocal at presentation, including hypothalamus
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DIA/Gs with anaplastic transformation
CaseAge @
DxAge @ GBM
outcome
Follow-up
(post-GBM)
Loh (2011)
2y 5y A, NR 11 yrs
Phi (2011)
7m 10y AWD 14 mos
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Clinical follow-up (6 months)
• s/p complete resection (required three craniotomies)
• Post-op ischemic changes with left sided weakness
• Chemotherapy w/o radiation therapy• No evidence of residual or recurrent
tumor
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Taratuto AL, Monges J, Lylyk P, et al (1984) Superficial cerebral astrocytoma attached to dura: report of six cases in infants. Cancer 54:2505–12.
Vandenberg SR, May EE, Rubinstein LJ, Herman MM, Perentes E, Vinores SA, Collins P, Park TS (1987) Desmo- plastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential (“desmoplastic infantile gangliogliomas”). Report of 11 cases of a distinctive embryonal tumor with favorable prognosis. J Neurosurg 66:58–71.
Duffner PK, Burger PC, Cohen ME, Sanford RA, Krischer JP, Elterman R, Aronin PA, Pullen J, Horowitz ME, Parent A, Martin P, Kun L (1994) Desmoplastic infantile gangliogliomas: an approach to therapy. Neurosurgery 34:583–589.
Al-Sarraj ST, Bridges LR (1996) Desmoplastic cerebral glioblastoma of infancy. British Journal of Neuropathology 10(2):215-219.
Kuchelmeister K, Steinhauser A, Korf B, et al (1996) Anaplastic desmoplastic infantile ganglioglioma. Clin Neuropathol 15:280.
De Munnynck K, Van Gool S, Van Calenbergh F, Demaerel P, Uyttebroeck A, Buyse G, et al (2002) Desmoplastic infantile ganglioglioma: a potentially malignant tumor? Am J Surg Pathol 26: 1515–1522.
Hoving, EW, Kros, JM, Groninger E, & Den Dunnen WFA (2008) Desmoplastic infantile ganglioglioma with a malignant course. J neurosurg Pediatrics 1:95-98.
Loh J-K, Lieu A-S, Chai C-Y, Howng S-L (2011) Malignant transformation of desmoplastic infantile ganglioglioma. Pediatric Neurology 45:135-137.
Phi JH, Koh EJ, Kim S-K, Park S-H, Cho B-K, & Wang K-C (2011) Desmoplastic infantile astrocytoma: recurrence with malignant transformation into glioblastoma: a case report. Childs Nerv Syst 27:2177-2181.
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